Hunterase ICV

Hunterase ICV (idursulfase-beta) is a recombinant human iduronate-2-sulfatase enzyme replacement therapy developed by GC Biopharma (formerly Green Cross) specifically to address the central nervous system (CNS) manifestations of Hunter syndrome (mucopolysaccharidosis type II). While intravenous enzyme replacement therapies like the standard Hunterase or Elaprase effectively treat systemic symptoms, they are unable to cross the blood-brain barrier in therapeutic concentrations. Hunterase ICV is delivered directly into the cerebral ventricles via an implanted Ommaya reservoir or similar intracerebroventricular (ICV) device. This approach allows the enzyme to reach brain cells and catabolize the accumulated glycosaminoglycans (GAGs) that lead to progressive neurocognitive decline. The drug received its first global approval in Japan in January 2021 for the treatment of patients with Hunter syndrome who exhibit or are at risk of neurocognitive symptoms.